Case reportPleural epithelioid hemangioendothelioma harboring CAMTA1 rearrangement
Introduction
Pleural epithelioid hemangioendothelioma (EHE) is very rare, and only around 20 cases have been reported in English literature [1], [2], [3], [4], [5], [6], [7], [8], [9], [10]. Based on these series of case reports, most patients with pleural EHE showed poor prognosis, less than 1 year of survival. Therefore, accurate diagnosis is very important, but often difficult in daily practice because of extremely low incidence and similar immunohistochemical profiling to other vascular tumor such as epithelioid hemangioma or epithelioid angiosarcoma [11]. Recently, CAMTA1 rearrangement has been introduced as a consistent genetic abnormality in EHEs of different anatomical locations [11], [12]. Herein, we report a 71-year-old man with pleural EHE confirmed by CAMTA1 rearrangement.
Section snippets
Case report
A 71-year-old man was referred to our hospital complaining of cough, dyspnea, fatigue and poor oral intake for 2 months. He also had weight loss of 8 kg for last 6 months. He was an ex-smoker (10 pack-year) and had no past medical history. He was a retired teacher and no history of asbestos exposure.
Laboratory findings were within normal limit, but mildly decreased total protein (5.1 g/dL), albumin (2.7 g/dL) and cholesterol (83 mg/dL) in serum. Conventional chest X-ray showed bilateral pleural
Discussion
Pleura is a rare anatomical site of EHE, and only around 20 cases have been reported in English literature [1], [2], [3], [4], [5], [6], [7], [8], [9], [10]. We summarized the cases of pleural EHE in Table 1, by modification from tables in previous reports by Lee et al. [6] and Marquez-Medina et al. [9]. While pulmonary EHE affects more commonly middle aged (mean age: about 40 years old) woman with no symptom, pleural EHE are more frequent in older adult males with clinical symptom associated
Conclusion
Pleural EHE is very rare disease entity with distinct clinical presentation and adverse outcome, comparing to its pulmonary counterpart. We report a 71-year-old man with pleural EHE confirmed by CAMTA1 rearrangement. Recently introduced CAMTA1 rearrangement by FISH may be useful ancillary test to confirm the diagnosis of pleural EHE, especially in small biopsy samples.
Conflict of interest statement
The authors declare no conflict of interest.
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